Paediatric surgery has been defined as the surgical care of the newborn,
the infant and the child. The specialty has grown in the United Kingdom as in
other parts of Europe and the world in a fairly close relationship to improving
social standards and medical care. Although in Edinburgh and Glasgow paediatric surgeons were established as specialists from the early 1920s, it was not
until 1950 in England and Wales that the number of paediatric surgeons exceeded
4, and in 1971 there are a total of 34 surgeons in the United Kingdom confining
their practice to paediatric surgery. Whilst few paediatric surgeons would claim
that the care of a child with a condition such as appendicitis demands skill and
expertise beyond that available to a general surgeon, paediatric surgeons, and
indeed most general surgeons, would agree that surgery in the newborn child and
in the infant suffering from the less common types of congenital abnormalities
demands not only special surgical experience but more importantly a team of
medical, nursing and technical specialists with sophisticated experience and equipment only available in a special unit.
At the turn of the century of every 1000 liveborn babies 140 died within
the first year of life; the main causes of this infant mortality rate were prematurity,
birth injury, respiratory difficulties and infections, and congenital abnormalities.
The infant mortality rate in 1969 was 18 and this enormous reduction has been achieved by improvements in social conditions affecting the pregnant mother, together
with improvements in obstetric care and the tremendously successful care of the
newborn child, developed during the past thirty years. As control of the other
lethal factors has been achieved the infant mortality from congenital abnormalities,
remaining unchanged at 4 per 1000, has assumed the role of major killer, and efforts
have been directed at reducing it. For example, in the Children's Hospital in
Sheffield in the late 1940s some 30 newborn children were operated on each year,
the number operated on now is more than 10 times as many.
Not all congenital abnormalities are incompatible with life and whilst
the correction of atresias of the alimentary tract or diaphragmatic hernias are
dramatic life saving procedures, congenital abnormalities of the cardiovascular
and central nervous systems are far more common and at the same time less immediately lethal. The correction of congenital heart disease during the first
three months of life has already altered the outlook to this type of abnormality
and will continue to do so for many years. Congenital abnormalities of the central nervous system, however, are far less dramatic in the toll they take of the
child in his first year of life and yet they are the commonest group seen at birth.
The most common lesion is a myelomeningocele with exposed spinal cord and consequent neuropathic changes in the lower limbs, pelvic floor and bladder and bowel
sphincters. Until 1957 no satisfactory treatment was available for the control of
the hydrocephalus which almost universally was an associated abnormality with
the severe lesions; there was little encouragement, therefore, for an attack on
the back lesion when it was almost certain that the child would die of progressive
hydrocephalus within a year or two. However, the introduction of a valve system
of ventriculo-cardiac drainage produced a method for control of progressive hydrocephalus and it soon became clear that an early covering of the exposed spinal cord
on the back would preserve such function as already was present in the lower limbs
and pelvis, whilst a negative approach leaving the closure to a slow process of
granulation and epithelialisation was almost certain to increase the degree of paraly¬
sis because of progressive damage to the exposed nerves. In many centres, therefore,
an early vigorous attack on the exposed lesion of the back was introduced and as
the interest of the paediatric surgeon in these cases became known to obstetricians
and paediatricians the number of cases referred for early treatment increased. In
an area such as Sheffield where the paediatric surgical unit drains a population of
approximately 3 million it could be expected that 120 cases would be referred each
year and this number represents almost the entire incidence of the condition in the
region. The estimated incidence of the condition is between 2 and 3 cases per
1000 live births and with a birthrate of 16 per thousand this would produce 40 cases
for each million population in each year.
It is too early as yet to decide whether the early treatment of the backs in
these children will effect the long term survival rates in the condition and certainly
it seems likely that some children die as a result of the surgical intervention who
might not have died had their backs been left to granulate; at the same time there
is no doubt that many children live much longer as a result of the early surgical
intervention and nor is there any doubt that having undertaken the primary treatment of the back in a child with myelomeningocele the surgeon and his unit must
hold themselves responsible for the continuing treatment of the child, whether
such treatment is necessary because of complications of the original surgical interference, or whether it is necessary to control the progress of those parts of the
disease which were not corrected at the original surgical interference,, Thus,
having closed the back it is necessary that progressive hydrocephalus should be
treated as required, that appropriate orthopaedic procedures should be carried out
in order to achieve and preserve the child's mobility, and that the upper urinary
tract should be protected and preserved from the devastation brought by the combination of urinary obstruction and infection resulting from the neuropathic bladder.
Not all this complicated care can be carried out by the same person who
originally closed the back and the general care of these children must depend on a
team which will include at least a paediatric surgeon, an orthopaedic surgeon and
a paediatrician. The particular interests of individuals will of course influence
the number of specialists taking an interest in these children,, An indication of
the amount of work that the continuing control of these children must bring to a
hospital is the fact that the Children's Hospital in Sheffield with an annual intake of
100 to 120 new cases for the past eight years, up to 40 of the total of 200 beds
available in the hospital are now occupied by children having treatment for spina
bifidao This does not include those children who are being cared for in hospitals
nearer their own homes or those in special long term orthopaedic hospitals,,
The condition thus places a heavy load on the hospital and its staff,,
Clearly medical and surgical care develops and efficiency of treatment improves,
the case material in a hospital will change; for instance at one time tuberculosis
of bones and joints accounted for a very large number of those children undergoing
surgery in a children's hospital and today it is practically unknown in the United
Kingdom. Similarly, infections such as osteomyelitis and empyema which at one
time formed a large proportion of the work in the children's hospital are now very
rare. These conditions have been excluded because they have been cured by advances in medical and surgical treatment. The surgical treatment of congenital abnormalities, however, and particularly the surgical treatment of myelomeningocele
often does not cure but only controls the condition, and in fact in these conditions
the paediatric surgeon frequently produces a new population which will demand repeated and often prolonged hospital ization. It would seem necessary, therefore, that those who are concerned in the treatment of congenital abnormalities
should consider the effects of that treatment. For the physician some consideration must be given to the quality of survival achieved as a result of extensive and
continuing courses of treatment. For the administrator and those who contribute
to the financing of the health services consideration should presumably be given
to the value of the results achieved; the financial resources of the health services
are not unlimited so there must be some competition for those resources and consequently one group of patients may suffer because of the concentration of funds
on another group. From a purely business outlook of cost and return there would
be little difficulty in deciding that the funds of the health services should be concentrated on those patients who will give the most productive return to the country
for the money that the country has spent on them.
Fortunately for the medical profession our decisions are not governed
entirely by financial considerations and the purpose of this thesis is to consider
the results achieved in the treatment of myelomeningocele and to discuss those ways
in which these results might be improved.